Document Detail


Allogeneic hematopoietic stem cell transplantation from an alternative stem cell source in Fanconi anemia patients: analysis of 47 patients from a single institution.
MedLine Citation:
PMID:  17053839     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We transplanted 47 patients with Fanconi anemia using an alternative source of hematopoietic cells. The patients were assigned to the following groups: group 1, unrelated bone marrow (N = 15); group 2, unrelated cord blood (N = 17), and group 3, related non-sibling bone marrow (N = 15). Twenty-four patients (51%) had complete engraftment, which was not influenced by gender (P = 0.87), age (P = 0.45), dose of cyclophosphamide (P = 0.80), nucleated cell dose infused (P = 0.60), or use of anti-T serotherapy (P = 0.20). Favorable factors for superior engraftment were full HLA compatibility (independent of the source of cells; P = 0.007) and use of a fludarabine-based conditioning regimen (P = 0.046). Unfavorable factors were > or = 25 transfusions pre-transplant (P = 0.011) and degree of HLA disparity (P = 0.007). Intensity of mucositis (P = 0.50) and use of androgen prior to transplant had no influence on survival (P = 0.80). Acute graft-versus-host disease (GVHD) grade II-IV and chronic GVHD were diagnosed in 47 and 23% of available patients, respectively, and infections prevailed as the main cause of death, associated or not with GVHD. Eighteen patients are alive, the Kaplan-Meyer overall survival is 38% at approximately 8 years, and the best results were obtained with related non-sibling bone marrow patients. Three recommendations emerged from the present study: fludarabine as part of conditioning, transplant in patients with < 25 transfusions and avoidance of HLA disparity. In addition, an extended family search (even when consanguinity is not present) seeking for a related non-sibling donor is highly recommended.
Authors:
C R de Medeiros; M A Bitencourt; J Zanis-Neto; E C P Maluf; D S Carvalho; C S Bonfim; V M Funke; D C Setubal; N Farah; R Pasquini
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Brazilian journal of medical and biological research = Revista brasileira de pesquisas médicas e biológicas / Sociedade Brasileira de Biofísica ... [et al.]     Volume:  39     ISSN:  0100-879X     ISO Abbreviation:  Braz. J. Med. Biol. Res.     Publication Date:  2006 Oct 
Date Detail:
Created Date:  2006-10-20     Completed Date:  2007-08-02     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8112917     Medline TA:  Braz J Med Biol Res     Country:  Brazil    
Other Details:
Languages:  eng     Pagination:  1297-304     Citation Subset:  IM    
Affiliation:
Serviço de Transplante de Medula Ossea, Hospital de Clínicas, Universidade Federal do Paraná, Rua General Carneiro 181, 80060-900 Curitiba, PR, Brazil. crdemedeiros@terra.com.br
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MeSH Terms
Descriptor/Qualifier:
Acute Disease
Adolescent
Adult
Child
Child, Preschool
Chronic Disease
Cyclophosphamide / therapeutic use
Fanconi Anemia / therapy*
Female
Graft vs Host Disease / diagnosis,  prevention & control
HLA Antigens / analysis
Hematopoietic Stem Cell Transplantation / methods*
Histocompatibility Testing
Humans
Immunosuppressive Agents / therapeutic use
Male
Multivariate Analysis
Retrospective Studies
Severity of Illness Index
Time Factors
Transplantation Conditioning / methods*
Transplantation, Homologous / immunology,  methods
Treatment Outcome
Chemical
Reg. No./Substance:
0/HLA Antigens; 0/Immunosuppressive Agents; 50-18-0/Cyclophosphamide

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